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Unmasking the Vampire’s Curse: The Medical Mystery of Porphyria

Unmasking the Vampire's Curse: The Medical Mystery of Porphyria

Porphyria: The Blood Disorder That Birthed Vampire Myths

Vampires have enthralled the darker corners of human imagination for centuries. From Dracula to today’s pop culture, these mythical creatures possess an enduring allure. Yet behind the supernatural mythos lies a real medical condition that may have fueled early vampire folklore.

Porphyria is a rare genetic disorder that can cause severe symptoms aligning uncannily with vampire traits. Sufferers develop extreme photosensitivity, reddened teeth and gums, skin lesions and anemia among other issues. Before modern medicine, these symptoms were not well understood. Those afflicted with porphyria bore resemblance to mythic vampires in both appearance and behavior.

One such individual was Mercy Brown, a young woman from 19th century New England. After Mercy passed away from disease, the townspeople suspected her corpse of vampirism when other family members fell ill. The exhumation of her body revealed porphyria-like signs taken as proof of vampirism by the superstitious community.

Mercy Brown and other cases like hers stoked the flames of vampiric folklore, especially in regions rife with superstition. While science has come to shed light on porphyria’s role, it is fascinating to reexamine these mysterious cases through a lens of both fact and fantasy. The connections between porphyria, mistaken as vampirism, and vampire lore run deeper than you may expect.

What is Porphyric Hemophilia?

Porphyria refers to a group of eight rare genetic blood disorders that affect the body’s ability to properly produce heme, the deep red pigment in hemoglobin. Heme allows red blood cells to carry and release oxygen.

The enzymes in the liver that produce heme are disrupted in porphyria patients. Each type of porphyria is caused by deficiencies in a specific enzyme involved in heme synthesis. When these enzymes are deficient, byproducts called porphyrins build up abnormally in the body. At high levels, porphyrins are toxic.

The most notorious type of porphyria connected to vampires is porphyria cutanea tarda (PCT). With PCT, there is a deficiency of uroporphyrinogen decarboxylase, the enzyme responsible for the fifth step in heme production. This buildup of uroporphyrins leads to severe photosensitivity of the skin when exposed to sunlight.

Porphyric Hemophilia

Other symptoms of porphyria cutanea tarda include:

  • Blistering, ulcerations, itching, and burning of exposed skin from sun sensitivity. This gave the impression vampires could not go out in daylight.
  • Increased hair growth on the forehead, cheeks and temples for a wolfish appearance.
  • Reddening of urine, which before modern plumbing may have been suspected as drinking blood.
  • Reddening of the teeth and gums to appear bloody.
  • Anemia from lack of hemoglobin causes fatigue, muscle weakness, and paleness resembling a corpse.
  • Increased heart rate and blood pressure.
  • Joint and back pain causing convulsions and spasms.

When taken together, the symptoms of PCT would have made sufferers seem supernaturally afflicted. The connections to mythical vampire traits are clear, especially before medical science understood porphyria.

While porphyria cutanea tarda can make sufferers appear vampire-like, it is important to note that porphyria is a medical condition caused by genetic enzyme deficiencies. It is not supernatural in origin. However, before modern science understood porphyria, people genuinely believed sufferers were vampires. The symptoms do create striking parallels between mythic vampire traits and medical porphyria patients. This is what allowed the disorder to be misinterpreted as vampirism throughout history in various parts of the world. But porphyria is not actually a “vampire disease” – it is a misunderstood condition that perpetuated vampire folklore and myths.

Cases of Porphyria Mistaken as Vampirism

While porphyria is rare today, it was more prevalent in isolated communities in the past. This allowed for multiple cases to stoke fears and folklore around vampires. Here are some prominent examples:

– In 18th century Serbia, several family members with porphyria were exhumed after death when villagers thought they became vampires. The corpses showed signs like reddish teeth and hair growth mistaken as supernatural.

– In 19th century Rhode Island, Mercy Brown’s exhumation revealed similar porphyria-like symptoms leading villagers to believe she was a vampire.

– Perhaps the most famous is Vlad the Impaler, the likely inspiration for Bram Stoker’s Dracula. Vlad may have exhibited porphyria symptoms, perpetuating vampiric legends long after his death.

These early cases capture how limited medical knowledge transformed porphyria into monsters in the eyes of superstitious communities. Without understanding what caused the disease’s symptoms, the afflicted were condemned as otherworldly evils that fed on people’s blood. These vivid historical examples show how folklore vampirism arose from disease.

Historical Cases of Alleged Vampirism Connected to Porphyria

While mistaken as supernatural, several prominent cases of alleged vampirism can be traced back to misunderstandings of porphyria.

Mercy Brown is a prime example

The Mercy Brown case in 19th century Rhode Island was one of the most infamous supposed vampire incidents in New England. In 1892, after many family members died of tuberculosis, locals feared the disease was spreading supernaturally.

Mercy Brown had passed away from tuberculosis years earlier, so her father allowed the exhumation of her corpse to quell the vampire rumors. When her body was unearthed, it showed little decomposition and blood seeping from the mouth, signaling to peasants that she was undead.

In reality, these symptoms match what occurs with porphyria victims, especially the blood from the mouth. However, amidst mass hysteria about consumption and limited medical knowledge, the villagers conflated Mercy’s disease symptoms with vampirism. They cut out her heart, burned it, mixed the ashes with water, and gave it to her ill brother to drink as an antidote. The vampire folk remedies did nothing, and Mercy’s brother died soon after.

While imagined as a vampire, Mercy Brown’s case provides a tragic example of porphyria symptoms being mistaken as supernatural due to hysteria about disease.

Key Facts related to Mercy Brown

  • Mercy Brown did die of tuberculosis in Exeter, Rhode Island in the late 19th century.
  • After multiple family members died of consumption, her father George Brown agreed to have her body exhumed in 1892 to investigate vampire rumors.
  • When her corpse was unearthed, contemporary accounts describe there being little decomposition and blood seeping from her mouth.
  • These symptoms matched prevailing folklore about vampire traits, though in reality align with porphyria.
  • The villagers did cut out Mercy’s heart, cremate it, and give the ashes mixed with water to her sick brother as a folk remedy.
  • Her brother died soon after of tuberculosis, showing the vampire “cure” was ineffective.

Arnold Paole a 18th century vampire

In the early 18th century, the region of Medveđa in central Serbia was at the epicenter of supposed vampire attacks that sparked great panic and shaped vampire lore across Europe. The frenzy began after the sudden death of peasant Arnold Paole in 1727. Paole had moved to the village months earlier, telling locals that he had been tormented by a vampire in another town before ultimately disposing of it. However, Paole died unexpectedly after falling from a haywagon in the fields.

In the ensuing months, villagers began perishing from a strange wasting disease marked by fever, cough, and blood discharge. As deaths mounted, locals grew suspicious that Paole had risen from the grave to prey on townspeople as a vampire. To confirm their fears, they exhumed Paole’s corpse a month after his funeral. Contemporary accounts describe the body seeming relatively untouched by decay, with fresh blood pooling around his mouth and flowing from his ears and nose.

While believed at the time to be signs of vampirism, these details match key symptoms of porphyria. However, with limited medical expertise, the townspeople interpreted the blood as evidence that Paole was undead. They drove a stake through his heart, at which point his body purportedly groaned and bled further, cementing the belief he had been a vampire.

In the following weeks, numerous other graves were exhumed, with normal post-mortem appearances taken as vampire activity. Hysteria grew as some bodies were dug up multiple times after showing continuing signs of blood discharge. Locals began staking and decapitating suspected vampire corpses including Paole’s and cremating them.

Word spread rapidly about the vampire infestation, which was verified by local Austrian administrators. They ordered further investigation that exhumed a total of 17 bodies. Their report conclusively declared the events as vampire attacks, confirming Paole as the first victim and initial vampire who triggered the contagion.

The perceived veracity of the Medveđa case made it renowned across Europe. It was cited in one of the earliest treatises on vampires in 1732, and fictional works like Tolstoy’s The Family of the Vourdalak drew direct inspiration from the incident. To this day, Paole remains one of the most influential cases cementing vampire lore in Eastern European folk beliefs.

Facts about the Arnold Paole case of Vampirism

  • – Arnold Paole was a Serbian peasant who died in 1727 after an accident while haying.
  • – Shortly after his death, villagers in the town of Medveđa began dying of a mysterious wasting illness.
  • – Rumors spread that Paole had become a vampire and was preying on the townspeople at night.
  • – In winter of 1727, villagers exhumed Paole’s corpse, which showed little decay and blood seeping from the mouth, eyes, and ears.
  • – These symptoms matched those of porphyria, but were taken as evidence Paole was undead.
  • – Villagers staked Paole’s heart, at which the body purportedly groaned and bled more.
  • – Over the next two months, 17 corpses were exhumed, with normal post-mortem changes seen as vampire activity.
  • – Bodies were staked, beheaded, and burned in efforts to stop the contagion.
  • – Austrian administrators verified the cases as vampire attacks centered on Paole as the initial vampire.
  • – The Arnold Paole case became renowned through treatises and fictional works across Europe.
  • – It was considered authoritative proof of vampires into the early 19th century.
  • – Paole’s case played a major role in cementing vampire folklore in Eastern Europe.

The Killer Vampire Gilles de Rais

Centuries before Arnold Paole, suspected vampire activities were attributed to French serial killer Gilles de Rais in the 15th century. De Rais was a nobleman who fought alongside Joan of Arc before retiring to his estates and descending into the brutal murders of hundreds of peasant children.

Before his execution in 1440 for Satanism and witchcraft, de Rais reportedly confessed to drinking his victims’ blood and studying alchemy to find the elixir of life. His motivations aligned closely with emerging myths around blood-drinking immortals and vampiric beasts.

After being hanged, his body was said to have not decomposed for several days. Blood seemingly seeped from his mouth and key porphyria symptoms were noted, such as bloated skin, sensitivity to sunlight, and overly red lips. At the time, these were taken as markers of his evil and vampiric ways.

Just as in Paole’s case centuries later, they likely signaled untreated porphyria exacerbated by de Rais’ alleged alchemy experiments with blood. However, the superstitions of the time conflated porphyria with vampirism, further reinforcing fears of diabolical vampires possessing dark abilities.

Though less famous than Paole, the shocking violence and vampire-like attributes ascribed to Gilles de Rais stoked early folklore. His case established links between vampirism, heresy, and demonic forces that persisted in European culture for generations afterward.

Vlad the Impaler

Vlad the Impaler

Another major influence on vampire legend was Vlad the Impaler, the 15th century prince of Wallachia known for brutal tactics against enemies. Vlad was believed to have impaled tens of thousands of victims and earned a fearsome reputation for cruelty during his reign. After his death, rumors spread that Vlad came back as a vampire-like creature and continued to wreak havoc. His patronym Dracula, meaning “son of the dragon” in Romanian, became associated with vampirism.

Bram Stoker cemented this connection in his iconic 1897 novel Dracula, which used Vlad as inspiration for the charismatic Count Dracula. Though there is no evidence the historical Vlad actually drank blood or possessed vampire traits, his warlike nature and propensity for violence primed imaginations to envision him as a vampire warlord after death. Along with cases like de Rais and Paole, the infamy of Vlad the Impaler fused together strands of folklore to coalesce into the vampire figures known today.

These cases and others show how limited medical knowledge transformed horror at illness into vampire legends. When viewed in historical context, alleged vampirism strongly links to medical conditions like porphyria that exhibit corpse-like symptoms and perpetuate folkloric fears.

In Video Games ( The Elder Scrolls )

Porphyria The Elder Scrolls Video Games

The acclaimed open-world fantasy video game series The Elder Scrolls has extensively incorporated porphyric illnesses into its vampire lore. Across multiple titles, the games’ rich universe adapts medical history to ground its vampires in a form of pseudo-science tied to porphyria. This reflects the significant imprint porphyria left on the origins of vampire folklore centuries ago.

Specifically, the disease Porphyric Hemophilia appears throughout several Elder Scrolls titles and their accompanying literature. With symptoms mirroring those of porphyria cutanea tarda, Porphyric Hemophilia is said to originate from the Daedric Prince Molag Bal. The disease creates skin lesions, photosensitivity, retractable fangs, and an overriding desire to drink blood.

In-game texts, like the book “On Porphyric Hemophilia,” detail how even minor consumption of vampire blood can spread Porphyric Hemophilia. As with historic misunderstandings around porphyria’s contagiousness, this reinforces vampire myths. However, it grounds the supernatural condition in real medical flaws.

Multiple Elder Scrolls protagonists, like the infected hero of Oblivion, struggle with Porphyric Hemophilia. While exaggerated for fantasy, this echo of past porphyria sufferers perpetuates its stigma. Yet it also evokes empathy for their plight. The disease is both a desired power and cursed affliction.

This nuanced incorporation of porphyria into Elder Scrolls vampirism reflects its complex scientific and cultural legacy. Even in a fictional world, porphyria’s presence in vampire origins is inextricable. Its symptoms lend an air of verisimilitude to fantastical blood-drinking immortals. By perpetuating the disease’s pseudoscientific links to vampires, the games uphold centuries of mythic conflation. Yet they also remind us of the real people who once endured porphyria’s mysterious afflictions alone.

Scientific Perspective on The Disease

Scientific Perspective on The Disease Porphyria

Before modern medicine, porphyria seemed a supernatural affliction. Those suffering exhibited sunlight sensitivity, disfigured skin, reddish teeth, and psychosis – symptoms ascribed to occult forces like vampirism. However, the enzymes underpinning porphyria are now understood.

Specifically, porphyria cutanea tarda (PCT) is tied to dysfunction of the enzyme uroporphyrilogen decarboxylase. This enzyme is critical in heme production, as it converts uroporphyrilogen III to coproporphyrilogen III in the biosynthesis pathway [2].

Without sufficient uroporphyrilogen decarboxylase activity, byproducts like uroporphyrinogen and other porphyrins accumulate. These compounds are excreted through urine and feces, making them red in color. They also deposit in the skin, causing severe photosensitivity and lesions when exposed to UV light.

Other symptoms arise secondary to these processes. For example, the skin scarring and reddish teeth due to porphyrin deposits. And psychosis potentially from neuron damage by the compounds.

While dramatic, these manifestations stemmed from enzymatic deficiency, not vampirism. Modern understanding of PCT’s molecular basis guides treatments like limiting iron absorption and porphyrin precursor drugs.

Rather than a supernatural curse, PCT is an enzyme disorder causing buildup of heme intermediates. Biochemistry dispelled the fiction of vampirism woven around porphyria centuries ago. Scientific insights transform mysticism into medicine.

[2] https://rarediseases.org/rare-diseases/porphyria-cutanea-tarda/

So Porphyria is not a Vampire disease?

Vampire working in a science lab for a cure

The whole vampire shtick started with porphyria centuries ago. Folks like Mercy Brown seemed to have the whole vampire package – the sun thing, nasty skin, fangs (well, reddish teeth). Without medical savvy, people blamed the supernatural.

But now science gets the credit for decoding porphyria. It’s not a weird hex – just funky enzymes. Still, we’re quick to slap “vampire” on strange new illnesses before doctors can sleuth them out. Patience, people! Gotta leave room for the experts to work their magic.

That said, vampire myths are tough to kill! Porphyria patients and spooky viruses are all over our books, shows and movies – the OG vampire inspo. But we gotta remember those bloodsucker stories were born from ignorance, not evidence. Fiction turned sickness into the romantic Dracula, not doctors.

So porphyria is a nice reminder – don’t leap to myths when medicine can unravel mysteries. Our brains love fantasies, but science brings the facts. Next time an illness seems supernatural, let’s chill until the docs figure it out. Stay wise, and leave the vampires to Hollywood!

Justin
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